Primary Pericardial Sarcoma with Right Atrial Invasion and Multiple Bilateral Pulmonary Metastases in a Patient with Hereditary Nonpolyposis Colorectal Cancer

Abstract EN

Primary tumours originating from the pericardium are extremely rare.

Previous studies have reported that these tumours account for only 6.7–12.8% of all mediastinal tumours with an overall prevalence of 0.001% to 0.007%.

The majority of these tumours are benign lipomas or pericardial cysts.

The most common pericardial malignancy is mesothelioma.

Sarcomas are soft-tissue mesenchymal malignancies originating from various parts of the body but are extremely rare in this area.

We report a case of a 52-year-old female who was diagnosed with a primary sarcoma with rhabdoid differentiation originating from the pericardium.

The patient presented to her GP with a four-week history of progressive dyspnea and chest pain on exertion.

Chest X-Ray demonstrated a prominent pericardial effusion and suspicious chest and pericardial lesions.

Biopsies of the effusion and primary tumour identified on FDG/PET scans revealed the diagnosis of primary undifferentiated sarcoma.

On thoracotomy, it was noted that the tumour had invaded the right atrium; therefore, pericardial window was aborted and a drain inserted instead.

The patient was then started on chemotherapy; however, progression soon occurred and the patient died within 4 months, suggesting there is urgent need for efficacious treatments for sarcomatous lesions.